Sofia Ahola MSc, PhD

Sofia Ahola

Department of Metabolism and Systems Science
Assistant Professor

Contact details

Address
IBR, 3rd floor
University of Birmingham
Edgbaston
Birmingham
B15 2TT
UK

Dr Sofia Ahola is an Assistant Professor in Mitochondrial Biology. Her research investigates how cells and tissues respond to mitochondrial dysfunction, with a particular focus on tissue-specific metabolic adaptation, mitochondrial disease and cardiomyopathy.

Qualifications

  • PhD in Medicine, University of Helsinki, Finland
  • MSc in Biosciences, University of Helsinki, Finland

Biography

Dr Sofia Ahola obtained her MSc in Biosciences from the University of Helsinki, Finland, where she subsequently completed her PhD in Medicine in the laboratory of Professor Anu Suomalainen Wartiovaara. Her doctoral research focused on mitochondrial disease mechanisms and metabolic remodelling, particularly in mitochondrial DNA maintenance-related myopathies. Her work explored therapeutic approaches, including exercise and dietary interventions, and contributed to the genetic diagnosis and characterisation of Leigh-like syndromes.

In 2015, she moved to Cologne, Germany, to join the laboratory of Professor Thomas Langer at the Max Planck Institute for Biology of Ageing and CECAD Research Centre. Her postdoctoral research focused on mitochondrial quality control, including the function of mitochondrial proteases and the proteolytic regulation of mitochondrial dynamics. Alongside this work, she developed a particular interest in how mitochondrial dysfunction reshapes cellular metabolism and stress responses in disease. Her work revealed how the regulation of mitochondrial dynamics supports mitochondrial biogenesis and hypertrophic growth of the heart during mitochondrial dysfunction. She also identified mechanisms that protect against ferroptosis in mitochondrial cardiomyopathy, establishing a link between mitochondrial dysfunction, cellular stress responses and metabolic remodelling.

In 2024, Dr Ahola joined the University of Birmingham as an Assistant Professor and established her independent research group in the Department of Metabolism and Systems Science. Her laboratory investigates the mechanisms that determine tissue-specific responses to mitochondrial dysfunction, combining mitochondrial biology with metabolic phenotyping and disease modelling.

Dr Ahola is also an investigator within the MRC Centre of Research Excellence in Mitochondrial Genome Therapeutics (MitoGT), where her group contributes expertise in tissue-specific metabolic responses and in vivo phenotyping of mitochondrial DNA disease.

Alongside her research, Dr Ahola has a longstanding interest in scientific illustration and visual communication. Her scientific illustrations have been used in research publications, reviews, presentations and cover artwork for scientific journals.

Teaching

Dr Ahola contributes to undergraduate and postgraduate teaching in cellular metabolism, mitochondrial biology and metabolic diseases. She teaches within the University of Birmingham Medical School and supervises undergraduate, postgraduate and doctoral research projects.

She is also a Personal Academic Tutor for medical students and contributes to research training and mentoring within the Department of Metabolism and Systems Science.

Postgraduate supervision

Dr Ahola welcomes enquiries from prospective PhD students interested in mitochondrial biology and disease. Areas of interest include mitochondrial quality control and dynamics, tissue-specific responses to mitochondrial dysfunction, metabolic adaptation, mitochondrial cardiomyopathy, oxidative stress and ferroptosis.

She has co-supervised PhD students at the Max Planck Institute for Biology of Ageing and currently supervises doctoral researchers at the University of Birmingham. Further PhD opportunities will arise through the MRC Centre of Research Excellence in Mitochondrial Genome Therapeutics (MitoGT), with projects focusing on tissue-specific metabolic responses to mitochondrial DNA disease.

Research

Dr Sofia Ahola’s research focuses on understanding how cells and tissues respond and adapt to mitochondrial dysfunction, and why these responses protect some tissues while contributing to disease in others. Her group is particularly interested in how mitochondrial dysfunction reshapes cellular metabolism, stress signalling and susceptibility to cell death, and how these processes contribute to mitochondrial and cardiac disease.

The group combines mechanistic studies in cellular and in vivo models with iPSC-derived cardiomyocytes and other disease-relevant cell types. A particular focus is on the heart, where maintaining mitochondrial function and metabolic flexibility is essential because of its exceptionally high energetic demands.

Current Research

  • Mitochondrial dysfunction, metabolic adaptation and cell fate

A major focus of the Ahola group is understanding how cells adapt when mitochondrial oxidative phosphorylation is impaired. The group investigates how mitochondrial dysfunction alters cellular metabolism, mitochondrial quality control and stress signalling, and how these responses differ between tissues.

An important part of this work examines how metabolic and antioxidant adaptations influence susceptibility to ferroptosis, an iron-dependent form of cell death driven by lipid peroxidation. By comparing cardiac, skeletal muscle and neuronal models, the group aims to identify mechanisms that determine why mitochondrial dysfunction can be tolerated in some cellular contexts but leads to progressive pathology in others.

  • Mitochondrial cardiomyopathy

The group investigates the mechanisms through which mitochondrial dysfunction causes cardiac disease. This work focuses on how the heart remodels its metabolism and mitochondrial homeostasis in response to impaired oxidative phosphorylation, and what determines whether these adaptations are protective or ultimately contribute to cardiac dysfunction.

Using genetic models of mitochondrial disease alongside iPSC-derived cardiomyocytes, the group studies metabolic remodelling, mitochondrial quality control, lipid metabolism and oxidative damage. The long-term aim is to identify mechanisms that can be therapeutically targeted to improve cardiac resilience in mitochondrial disease.

  • MRC CoRE in Mitochondrial Genome Therapeutics (MitoGT)

Dr Ahola is part of the MRC Centre of Research Excellence in Mitochondrial Genome Therapeutics (MitoGT), a major multidisciplinary programme developing new approaches to understand and treat diseases caused by mutations in mitochondrial DNA.

Within MitoGT, the Ahola group contributes to defining the cell- and tissue-specific vulnerabilities that determine the progression and clinical manifestations of mtDNA disease. Her research focuses particularly on metabolic phenotyping and in vivo models to understand why different tissues show markedly different susceptibility or resilience to mitochondrial dysfunction. By investigating tissue-specific metabolic rewiring, substrate utilisation and physiological responses to pathogenic mtDNA mutations, the group aims to identify mechanisms that determine disease severity and progression and reveal potential biomarkers and therapeutic targets.

Research interests

The group’s broader research interests include mitochondrial disease, mitochondrial cardiomyopathy, oxidative phosphorylation, metabolic remodelling, mitochondrial quality control, stress signalling, ferroptosis and lipid peroxidation, and iPSC-based disease modelling.

Other activities

Sofia Ahola paints and draws scientific illustrations and some of her work is shown in her webpage; www.sofiaahola.com 

Publications

Recent publications

Article

Ahola, S, Pazurek, LA, Mayer, F, Lampe, P, Hermans, S, Becker, L, Amarie, OV, Fuchs, H, Gailus-Durner, V, Angelis, MHD, Riedel, D, Nolte, H & Langer, T 2024, 'Opa1 processing is dispensable in mouse development but is protective in mitochondrial cardiomyopathy', Science Advances, vol. 10, no. 31, eadp0443. https://doi.org/10.1126/sciadv.adp0443

Rivera-Mejías, P, Narbona-Pérez, ÁJ, Hasberg, L, Kroczek, L, Bahat, A, Lawo, S, Folz-Donahue, K, Schumacher, A-L, Ahola, S, Mayer, FC, Giavalisco, P, Nolte, H, Lavandero, S & Langer, T 2023, 'The mitochondrial protease OMA1 acts as a metabolic safeguard upon nuclear DNA damage', Cell Reports, vol. 42, no. 4, 112332. https://doi.org/10.1016/j.celrep.2023.112332

Ahola, S, Rivera mejías, P, Hermans, S, Chandragiri, S, Giavalisco, P, Nolte, H & Langer, T 2022, 'OMA1-mediated integrated stress response protects against ferroptosis in mitochondrial cardiomyopathy', Cell Metabolism, vol. 34, no. 11, pp. 1875-1891.e7. https://doi.org/10.1016/j.cmet.2022.08.017

Sprenger, HG, Wani, G, Hesseling, A, König, T, Patron, M, MacVicar, T, Ahola, S, Wai, T, Barth, E, Rugarli, EI, Bergami, M & Langer, T 2019, 'Loss of the mitochondrial iii-AAA protease YME1L leads to ocular dysfunction and spinal axonopathy.', EMBO Molecular Medicine. https://doi.org/10.15252/emmm.201809288

Buzkova, J, Nikkanen, J, Ahola, S, Hakonen, AH, Sevastianova, K, Hovinen, T, Yki-Järvinen, H, Pietiläinen, K, Lönnqvist, T, Velagapudi, V, Carroll, C & Suomalainen, A 2018, 'Metabolomes of mitochondrial diseases and inclusion body myositis patients: treatment targets and biomarkers.', EMBO Molecular Medicine. https://doi.org/10.15252/emmm.201809091

Nikkanen, J, Forsström, S, Euro, L, Paetau, I, Kohnz, RA, Wang, L, Chilov, D, Viinamäki, J, Roivainen, A, Marjamäki, P, Liljenbäck, H, Ahola, S, Buzkova, J & Suomalainen, A 2016, 'Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism.', Cell Metabolism. https://doi.org/10.1016/j.cmet.2016.01.019

Ahola, S, Auranen, M, Isohanni, P, Niemisalo, S, Urho, N, Buzkova, J, Velagapudi, V, Lundbom, N, Hakkarainen, A, Muurinen, T, Piirilä, P, Pietiläinen, KH & Suomalainen, A 2016, 'Modified Atkins diet induces subacute selective ragged-red-fiber lysis in mitochondrial myopathy patients.', EMBO Molecular Medicine. https://doi.org/10.15252/emmm.201606592

Ahlqvist, KJ, Leoncini, S, Pecorelli, A, Wortmann, SB, Ahola, S, Forsström, S, Guerranti, R, De, FC, Smeitink, J, Ciccoli, L, Hämäläinen, RH & Suomalainen, A 2015, 'MtDNA mutagenesis impairs elimination of mitochondria during erythroid maturation leading to enhanced erythrocyte destruction.', Nature Communications. https://doi.org/10.1038/ncomms7494

Ahola, S, Isohanni, P, Euro, L, Brilhante, V, Palotie, A, Pihko, H, Lönnqvist, T, Lehtonen, T, Laine, J, Tyynismaa, H & Suomalainen, A 2014, 'Mitochondrial EFTs defects in juvenile-onset Leigh disease, ataxia, neuropathy, and optic atrophy.', Neurology. https://doi.org/10.1212/WNL.0000000000000716

Guse, K, Suzuki, M, Sule, G, Bertin, TK, Tyynismaa, H, Ahola-Erkkilä, S, Palmer, D, Suomalainen, A, Ng, P, Cerullo, V, Hemminki, A & Lee, B 2012, 'Capsid-modified adenoviral vectors for improved muscle-directed gene therapy', Human Gene Therapy, vol. 23, no. 10, pp. 1065-1070. https://doi.org/10.1089/hum.2012.003

Tyynismaa, H, Sun, R, Ahola-Erkkilä, S, Almusa, H, Pöyhönen, R, Korpela, M, Honkaniemi, J, Isohanni, P, Paetau, A, Wang, L & Suomalainen, A 2012, 'Thymidine kinase 2 mutations in autosomal recessive progressive external ophthalmoplegia with multiple mitochondrial DNA deletions', Human Molecular Genetics, vol. 21, no. 1, pp. 66-75. https://doi.org/10.1093/hmg/ddr438

Editorial

Ahola, S 2024, 'Editorial: Mitochondrial bioenergetics and metabolism: implication for human health and disease', Frontiers in Molecular Biosciences, vol. 11, 1468758. https://doi.org/10.3389/fmolb.2024.1468758

Other contribution

Ahola, S, Langer, T & MacVicar, T 2019, Mitochondrial Proteolysis and Metabolic Control. https://doi.org/10.1101/cshperspect.a033936

Review article

Ahola, S 2026, 'Mitochondria setting the stage for ferroptosis', Trends in Endocrinology and Metabolism. https://doi.org/10.1016/j.tem.2026.06.006

Ahola, S & Langer, T 2024, 'Ferroptosis in mitochondrial cardiomyopathy', Trends in Cell Biology, vol. 34, no. 2, pp. 150-160. https://doi.org/10.1016/j.tcb.2023.06.002

View all publications in research portal